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Background: Central line-associated bloodstream infections (CLABSIs) are major sources of morbidity, death, and healthcare costs in patients who receive home parenteral nutrition (HPN). The majority of HPN-dependent children in southern Israel reside in poor communities with substandard living conditions, which creates significant challenges for the safe provision of HPN. We developed a pilot intervention that aimed to reduce the rates of CLABSI and central venous catheter (CVC) replacements in this vulnerable population in our region., Methods: Between 2012 and 2014, all HPN-dependent children with intestinal failure who were treated in our center, received HPN through a Hickman catheter, and experienced at least 1 previous CLABSI episode participated in the intervention. The intervention included home visits to assess the caregivers' CVC-handling technique, instillation of prophylactic ethanol lock solution, and the convening of regular multidisciplinary staff debriefings. We calculated CLABSI and CVC-replacement rates before and after the intervention., Results: Eight patients who served as their own historical controls were included in the intervention (total of 2544 catheter-days during the intervention period). The mean CLABSI rate decreased from 9.62 to 0.79 CLABSI episodes per 1000 catheter-days; the CVC-replacement rate decreased from 2.5 to 1.2 replacements per 1000 catheter-days in the preintervention and intervention periods respectively. The median hospital length of stay and individual monthly cost of medical care decreased compared to those found in the preintervention period., Conclusions: The results of this study offer a proof of concept for a strategy to reduce CLABSI rates in pediatric patients who reside in remote and low-resource environments and are undergoing HPN., (© The Author(s) 2019. Published by Oxford University Press on behalf of The Journal of the Pediatric Infectious Diseases Society. All rights reserved. For permissions, please e-mail: journals.permissions@oup.com.)

Aim: This study investigated the seasonality of birth in children diagnosed with coeliac disease (CD) at a tertiary University hospital in Southern Israel., Methods: This was a population-based retrospective time series analysis study from January 1988 to December 2014. There were 308 903 live births at Soroka University Medical Centre during the study period and 699 were diagnosed with CD. We combined three databases covering births, CD diagnoses and weather indices. The daily proportion of births that resulted in CD for the different four seasons and high seasons were compared to the weather indices on the day of birth using negative binomial regression., Results: Statistically significant associations were found between the season of birth and the rate of CD, with autumn births being associated with a higher risk for the development of CD than births during the summer, with an incidence ratio of 1.22. The association was further increased when the defined summer and autumn high seasons were used, with an incidence ratio of 1.40. No association was found between CD and the mean temperature and global radiation., Conclusion: Coeliac disease was associated with birth during the autumn and the autumn high season posed an even more significant risk factor., (©2018 Foundation Acta Paediatrica. Published by John Wiley & Sons Ltd.)

Purpose: To report the incidence and severity of the oral and dental manifestations associated with congenital insensitivity to pain with anhidrosis (CIPA)., Methods: Young children with CIPA underwent orofacial examination. The tongue, lips, and buccal mucosa were examined for soft tissue disorder. Missing and luxated teeth were recorded., Results: Twenty four patients (14 males and 10 females, mean age 60 months, range 9-144 months) with CIPA showed moderate to severe self-mutilation. Oral self-mutilation, such as biting injuries and scarring of soft tissues (tongue, lip, and buccal mucosa) were found in all patients. Fingertip biting was also found in most patients. Among infant patients, the mutilation was typically characterized by decubital ulcers of the tongue. Many edentulous areas due to previously extracted teeth were also found., Conclusions: Early diagnosis and specific dental care for patients with CIPA can be helpful in prevention of the fingertip biting and orofacial manifestations seen in this disorder.

Congenital insensitivity to pain with anhidrosis (CIPA), a rare and severe disorder, comprises absence of sensation to noxious stimuli, inability to sweat, and recurrent episodes of hyperthermia. It has a relatively high prevalence in the consanguineous Israeli-Bedouins. Clinical studies of 28 patients are reported here. Using the linkage analysis approach, we linked the disease in 9 of 10 unrelated Israeli-Bedouin families with CIPA to the TrkA gene, which encodes the receptor for nerve growth factor. In one family, linkage was excluded, implying that another gene, yet unidentified, is involved. Two new mutations in the tyrosine kinase domain of the TrkA gene were identified in our CIPA patients: a 1926-ins-T in most of the southern Israeli-Negev CIPA patients, and a Pro- 689-Leu mutation in a different isolate of Bedouins in northern Israel. Eight prenatal diagnoses were made in the southern Israeli-Negev Bedouins, two by linkage analysis and six by checking directly for the 1926-ins-T mutation. Three polymorphisms in the TrkA protein kinase encoding domain were also observed., (Copyright 2000 Wiley-Liss, Inc.)